t(17;21)(q11.2;q22) as a sole aberration in acute myelomonocytic leukemia

Case report of a translocation : t(17;21)(q11.2;q22) as a sole aberration in acute myelomonocytic leukemia

Complex systems and the technology of variability analysis

Characteristic patterns of variation over time, namely rhythms, represent a defining feature of complex systems, one that is synonymous with life. Despite the intrinsic dynamic, interdependent and nonlinear relationships of their parts, complex biological systems exhibit robust systemic stability. Applied to critical care, it is the systemic properties of the host response to a physiological insult that manifest as health or illness and determine outcome in our patients. Variability analysis provides a novel technology with which to evaluate the overall properties of a complex system. This review highlights the means by which we scientifically measure variation, including analyses of overall variation (time domain analysis, frequency distribution, spectral power), frequency contribution (spectral analysis), scale invariant (fractal) behaviour (detrended fluctuation and power law analysis) and regularity (approximate and multiscale entropy). Each technique is presented with a definition, interpretation, clinical application, advantages, limitations and summary of its calculation. The ubiquitous association between altered variability and illness is highlighted, followed by an analysis of how variability analysis may significantly improve prognostication of severity of illness and guide therapeutic intervention in critically ill patients

Cyclosporin A as Immunosuppressive Treatment Option for Patients with Refractory Auto-Immune Thrombocytopenic Purpura.

Abstract The treatment of auto-immune thrombocytopenic purpura (AITP) remains unsatisfactory in patients refractory to first-line management such as corticosteroids and/or splenectomy. Those patients usually require unacceptably high doses of corticosteroids to maintain a safe platelet count and thus have refractory AITP. Relativelly new immunosupressive treatment modality is cyclosporin A (CsA) and no large studies involving these drug have been conducted. We treated 7 refractory AITP patients with CsA. Their platelet count were without any therapy below 20x109/L and mostly they had signs of subcutaneous and mucosal bleeding. In 6/7 patients splenectomy was performed earlier. In order to maintain »safe« platelet count, they all needed methylprednisolon (MP), at least 32 mg/daily. So at the time treatment with CsA began, all patients were on MP. During next months MP dosage was tapered or withdrew, according to patients platelet count and treatment efficacy. At the endpoint, in 4/7 patients complete remission (CR) was achieved and later CsA was slowly ceased. 3/7 patients are currently in partial remission (PR) of the disease. Also one of them who has more than 30 years history of the disease is in stable PR, on low dose CsA and low dose MP maintanance therapy. Her platelet count is well above 20x109/L. Among CsA treatment related side effects painful lower limb edema was most frequent. Based on our experience we suggest, that CsA should be recommended in refractory (chronic) AITP patients, because it shows long-term efficacy and good safety profile and is able to sustain remission at low doses or even after CsA discontinuation.</jats:p